Legg-Calvé-Perthes, disease

[OMIM 150 600]

(Infant primitive Osteochondritis of the hip)

Common condition: 1 child on 2 000 in Europe, and is occasionally bilateral (10% of cases). Progressive avascular necrosis of the femoral head following the interruption of the vascularization to the proximal femoral epiphysis. The etiology is unknown: a mechanical factor (repetitive strain injury) is suspected, but a mutation in the COL2A1 gene (12q12-q13.2) is found in familial cases. It occurs typically in boys (sex ratio of 8:1) aged  4 to 8 years, hyperactive, small for age and presenting with delayed bone maturation. It manifests itelf clinically as a limp and/or a painful hip (but sometimes also by a knee pain). Medical imaging makes the diagnosis and clarifies the evolutionary stage:

-        stage 1: condensation of the femoral head (3-6 months);

-        stage 2: fragmentation (10 months);

-        stage 3: reconstruction (3 years) that can lead to a complete cure.

-        stage 4: deformation sequellae (coxa magna, future osteoarthritis).


Therapeutic management depends on the age of the child, the extension of the lesions, the existence of joint stiffness and the family context. Orthopaedic methods aim at unloading the hip, guiding the reconstruction of the femoral head, by centering the latter in the acetabulum and redistributing pressure on the epiphysis. Severe forms require surgical treatment; they often concern children aged over 6 years who already have some stiffening of the hip that should absolutely be corrected (by traction in bed for 3 weeks) before the surgery. This one must be early (before the 8th month of evolution). Two surgical procedures are commonly proposed:

-        varisation osteotomy of the femur, to redirect the femoral head into the acetabulum, in forms where the femoral head is little distorted

-        triple osteotomy of the pelvis (Salter operation) to reshape the acetabulum to contain the femoral head

Some major forms may require the combination of femoral varisation osteotomy and triple osteotomy of the pelvis.


Anesthetic implications:

lengthy bleeding surgery and painful postoperatively. Femoral nerve block or lumbar epidural block.


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Updated: Fbruary 2019